Divisions of Pulmonary, Critical Care and Sleep Medicine, USA; McGovern Medical School, The University of Texas Health Science Center at Houston, TX, 77030, USA.
Divisions of Pulmonary, Critical Care and Sleep Medicine, USA; Spectrum Health-Michigan State University College of Human Medicine, Grand Rapids, MI, 49503, USA.
Respir Med. 2019 Aug;155:86-96. doi: 10.1016/j.rmed.2019.07.011. Epub 2019 Jul 11.
Developmental lung anomalies represent a heterogeneous group of diverse, yet related abnormalities that involve the lung parenchyma, pulmonary vasculature or a combination of both-which usually present prenatally and in early childhood. However, a substantial number of cases go unnoticed during childhood and present either incidentally or with recurrent respiratory infections progressing into adulthood. Defective development of the tracheobronchial tree and the pulmonary vasculature are proposed to cause these developmental anomalies. Encountering these lung anomalies in adults is a diagnostic challenge given their rarity and they are often mistaken as other serious pathological conditions, resulting in unnecessary diagnostic tests and procedures. The developmental lung anomalies in adults can be broadly divided into three categories: bronchopulmonary anomalies encompassing congenital bronchial atresia, bronchogenic cysts, congenital lobar emphysema and congenital pulmonary airway malformations. Vascular anomalies include pulmonary agenesis-aplasia- hypoplasia complex, unilateral absence of the pulmonary artery, pulmonary artery sling, partial anomalous pulmonary venous return, pulmonary venous varix and pulmonary arteriovenous malformations. Finally combined lung parenchymal-vascular anomalies of the lung involve hypogenetic lung (scimitar) syndrome and bronchopulmonary sequestration. This article discusses the spectrum of these developmental anomalies of the lung, their etiopathogenesis, clinical and radiographic presentations in adults and management in brief.
发育性肺异常是一组异质性的、多样化的肺实质、肺血管或两者结合的相关异常，这些异常通常在产前和幼儿期表现出来。然而，相当数量的病例在儿童期未被发现，或者表现为偶发性或复发性呼吸道感染，直至成年。气管支气管树和肺血管的发育缺陷被认为是导致这些发育性异常的原因。由于这些肺异常在成人中较为罕见，而且常被误诊为其他严重的病理情况，导致不必要的诊断性检查和程序，因此在成人中遇到这些肺异常是一项具有挑战性的诊断。成人的发育性肺异常可大致分为三类：支气管肺异常包括先天性支气管闭锁、支气管源性囊肿、先天性大叶性肺气肿和先天性肺气道畸形。血管异常包括肺发育不全-发育不良-发育不良综合征、单侧肺动脉缺如、肺动脉吊带、部分肺静脉异常回流、肺静脉瘤和肺动静脉畸形。最后，肺实质-血管联合异常包括肺发育不良（镰状）综合征和支气管肺隔离症。本文讨论了这些肺发育异常的范围、病因、成人的临床表现和影像学表现以及简要的治疗方法。
